Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease

ABSTRACT

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.

Areas covered: We reviewed EGPA clinical presentation, focusing on clinical manifestations at three different phases of the disease: 1/before the development of overt vasculitis, 2/at vasculitis diagnosis and 3/during the long-term follow-up. An update on current classification criteria and recent therapeutic advances has been provided as well.

Expert opinion: Asthma, chronic rhinosinusitis and blood eosinophilia could anticipate the overt vasculitis for years. An atopic background may be present in a subset of patients (25-30%), while ANCA presence varies between 10 and 40%. Systemic vasculitis rapidly occurs and clinical features demonstrating vasculitis processes (neuropathy, purpura, scleritis, alveolar hemorrhage and glomerulonephritis) develop along with systemic symptoms (50%). After vasculitis resolution, asthma remains severe in up to 50% of patients and incidence of isolated-asthma and rhinosinus exacerbations remains constantly high. Different sets of classification criteria have been published so far, and DCVAS diagnostic criteria will be presented soon. Interleukin-5 blockers seem to be promising to control the disease and to spare corticosteroids.